Cochlear pathology of the circling mouse: A new mouse model of DFNB6

Won Ho Chung, Ki Ryung Kim, Yang Sun Cho, Do Yeon Cho, Ji Hwan Woo, Zae Young Ryoo, Kyoung In Cho, Sung Hwa Hong

Research output: Contribution to journalArticlepeer-review

25 Scopus citations

Abstract

Conclusion. The circling mouse (cir/cir) has phenotypes which follow the pattern of neuroepithelial defects of deafness from 10 days after birth. The cir mouse is defective in Tmie gene, the function of which should be further elucidated. Objectives. We previously reported a recessive mutation of deafness called circling mice (cir/cir). The present study focused on investigating phenotypes and histological findings of the cochlea in circling mice with respect to age. Materials and methods. In order to analyze cochlear pathology over time, five different age groups of circling mice were examined (10, 18, 21, 35, and 90 days old). The organs of Corti and spiral ganglion neurons in basal and middle turns were evaluated. Results. The pathology of the organ of Corti followed the pattern of neuroepithelial defects. Hair cells in organs of Corti had degenerated in circling mice at 10 days old, in a time-dependent manner. Scanning electron microscopy (SEM) showed that stereociliary bundles were irregular in size and had shortened at 10 days, and that this degeneration was complete at 21 days. The number of spiral ganglion neurons significantly reduced with age. RT-PCR analysis indicated that the transmembrane inner ear gene (Tmie) was absent in various organs in circling mice.

Original languageEnglish
Pages (from-to)244-251
Number of pages8
JournalActa Oto-Laryngologica
Volume127
Issue number3
DOIs
StatePublished - 2007

Keywords

  • Animal model
  • Deafness
  • Hearing loss
  • Neuroepithelial defects
  • Organ of Corti
  • Recessive gene
  • Tmie

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